Brainstem auditory evoked potentials in Wilson's disease

Twelve patients with Wilson's disease, aged 11–25 years, underwent brainstem auditory evoked potential (BAEP) study. The results were correlated to clinical, neuroradiological and laboratory data. Ten had prominent to severe neurological manifestations, suggestive of involvement of one or sever...

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Veröffentlicht in:Journal of the neurological sciences 1990-02, Vol.95 (2), p.163-169
Hauptverfasser: Butinar, Dušan, Trontelj, Jože V., Khuraibet, Adnan J., Khan, Riyad A., Hussein, Jassim M., Shakir, Ra'ad A.
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container_end_page 169
container_issue 2
container_start_page 163
container_title Journal of the neurological sciences
container_volume 95
creator Butinar, Dušan
Trontelj, Jože V.
Khuraibet, Adnan J.
Khan, Riyad A.
Hussein, Jassim M.
Shakir, Ra'ad A.
description Twelve patients with Wilson's disease, aged 11–25 years, underwent brainstem auditory evoked potential (BAEP) study. The results were correlated to clinical, neuroradiological and laboratory data. Ten had prominent to severe neurological manifestations, suggestive of involvement of one or several CNS structures, and 2 were neurologically free. All had evidence of abnormal copper metabolism, and 8 had CT scan evidence of brain atrophy, or hypodense areas in basal ganglia, or both. The 2 patients without neurological manifestations as well as one with neurological signs had normal BAEP. One patient with neurological signs had increased N1 latency due to cochlear hearing loss, but normal interpeak intervals, while 8 of 10 patients with prominent neurological symptoms and signs had abnormal BAEPs (prolongation of NIII-NV interpeak interval). The value of NIII-NV interpeak interval correlated with the number of different neurological signs (neurological score) attributable to involvement of different CNS structures ( r = 0.64 at P = 0.001). Abnormal BAEPs do not seem to be an early finding in Wilson's disease.
doi_str_mv 10.1016/0022-510X(90)90239-J
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Electric activity recording</topic><topic>Evoked Potentials, Auditory</topic><topic>Female</topic><topic>Hepatolenticular Degeneration - physiopathology</topic><topic>Humans</topic><topic>Investigative techniques, diagnostic techniques (general aspects)</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Neurological manifestations</topic><topic>Reaction Time</topic><topic>Silent CNS lesions</topic><topic>Wilson's disease</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Butinar, Dušan</creatorcontrib><creatorcontrib>Trontelj, Jože V.</creatorcontrib><creatorcontrib>Khuraibet, Adnan J.</creatorcontrib><creatorcontrib>Khan, Riyad A.</creatorcontrib><creatorcontrib>Hussein, Jassim M.</creatorcontrib><creatorcontrib>Shakir, Ra'ad A.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Journal of the neurological sciences</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Butinar, Dušan</au><au>Trontelj, Jože V.</au><au>Khuraibet, Adnan J.</au><au>Khan, Riyad A.</au><au>Hussein, Jassim M.</au><au>Shakir, Ra'ad A.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Brainstem auditory evoked potentials in Wilson's disease</atitle><jtitle>Journal of the neurological sciences</jtitle><addtitle>J Neurol Sci</addtitle><date>1990-02</date><risdate>1990</risdate><volume>95</volume><issue>2</issue><spage>163</spage><epage>169</epage><pages>163-169</pages><issn>0022-510X</issn><eissn>1878-5883</eissn><coden>JNSCAG</coden><abstract>Twelve patients with Wilson's disease, aged 11–25 years, underwent brainstem auditory evoked potential (BAEP) study. The results were correlated to clinical, neuroradiological and laboratory data. Ten had prominent to severe neurological manifestations, suggestive of involvement of one or several CNS structures, and 2 were neurologically free. All had evidence of abnormal copper metabolism, and 8 had CT scan evidence of brain atrophy, or hypodense areas in basal ganglia, or both. The 2 patients without neurological manifestations as well as one with neurological signs had normal BAEP. One patient with neurological signs had increased N1 latency due to cochlear hearing loss, but normal interpeak intervals, while 8 of 10 patients with prominent neurological symptoms and signs had abnormal BAEPs (prolongation of NIII-NV interpeak interval). The value of NIII-NV interpeak interval correlated with the number of different neurological signs (neurological score) attributable to involvement of different CNS structures ( r = 0.64 at P = 0.001). 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subjects Adolescent
Adult
Biological and medical sciences
Brain Stem - physiopathology
Brainstem auditory evoked potentials
Child
CT findings
Electrodiagnosis. Electric activity recording
Evoked Potentials, Auditory
Female
Hepatolenticular Degeneration - physiopathology
Humans
Investigative techniques, diagnostic techniques (general aspects)
Male
Medical sciences
Neurological manifestations
Reaction Time
Silent CNS lesions
Wilson's disease
title Brainstem auditory evoked potentials in Wilson's disease
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